PURPLE (atrophie blanche): clinical, histological and immunological study of twelve patients

Abstract

Objectives To investigate clinical, serologic, histopathologic and immunopathologic markers of PURPLE (painful purpuric ulcers with reticular pattern of lower extremities) or atrophie blanche.Design Twelve consecutive patients affected with idiopathic PURPLE were studied in the period 1992–1996. Patients with systemic diseases correlated to PURPLE were excluded from the study.Subjects All twelve patients were hospitalized at the Istituto Dermopatico Immacolata in Rome.Results Various and dishomogeneous immunological alterations were detected. Circulating immune complexes, increased serum levels of anticardiolipin and antinuclear antibodies, reduced serum complement levels, and deposition of immunoreactants in dermal vessels, were found in a limited number of patients. The results were not statistically significant. All the patients showed very similar histopathological aspects, characterized by microvascular thromboses, endothelial swelling and segmental hyalinization of small dermal vessels.Conclusion PURPLE is a thrombogenic vasculopathy in which unspecified immunological and laboratory changes are present. The recently suggested pathogenetic role of anticardiolipin and protein C deficiency in the disorder is not confirmed by this study.